Pediatric rare lung disease (PRLD) is a term that refers to a heterogeneous group of rare disorders in children. In recent years, this field has experienced significant progress marked by scientific discoveries, multicenter and interdisciplinary collaborations, and efforts of patient advocates. Although genetic mechanisms underlie many PRLDs, pathogenesis remains uncertain for many of these disorders. Furthermore, epidemiology and natural history are insufficiently defined, and therapies are limited. To develop strategies to accelerate scientific advancement for PRLD research, the NHLBI of the National Institutes of Health convened a strategic planning workshop on September 3 and 4, 2015...

OBJECTIVE: To improve systems of care to advance implementation of the U.S. Preventive Services Task Force recommendations for childhood obesity treatment (i.e., clinicians offer/refer children with obesity to intensive, multicomponent behavioral interventions of >25 h over 6 to 12 months to improve weight status) and to expand payment for these services. METHODS: In July 2015, 43 cross-sector stakeholders attended a conference supported by the Agency for Healthcare Research and Quality, American Academy of Pediatrics Institute for Healthy Childhood Weight, and The Obesity Society...

OBJECTIVE: To test the feasibility of a randomized-controlled trial measuring the impact of an adapted mindfulness-based intervention (MBI) in female adolescents with chronic pain. METHODS: This was a single center, single-blind, prospective, experimental, longitudinal trial conducted in a pediatric tertiary care center. Participants had a history of chronic pain during at least three months. They were randomized into an intervention group or a wait-list control group...

In principle, proton therapy offers a substantial clinical advantage over the conventional photon therapy. This is because of the unique depth-dose characteristics of protons, which can be exploited to achieve significant reductions in normal tissue doses proximal and distal to the target volume. These may, in turn, allow escalation of tumor doses, greater sparing of normal tissues, thus potentially improving local control and survival while at the same time reducing toxicity and improving quality of life. Protons, accelerated to therapeutic energies ranging from 70 to 250MeV, typically with a cyclotron or a synchrotron, are transported to the treatment room where they enter the treatment head mounted on a rotating gantry...

BACKGROUND: Familial hypercholesterolemia (FH), the prevalent monogenic form of hypercholesterolemia, carries the risk of premature coronary heart disease. Lipoprotein-apheresis is established in children with severe dyslipidemia. We present 3 siblings with a negative/negative residual low-density lipoprotein (LDL) receptor mutation (p.Trp577Arg), unresponsive to drug treatment. OBJECTIVE: Intensified lipoprotein-apheresis is well tolerated and results in permanently low lipid values without harming the health-related quality of life in children...

BACKGROUND: Fatigue is common in patients with JIA and affects daily life negatively. We assessed the presence and severity of fatigue in patients with JIA, including factors presumed associated with fatigue (e.g., disease activity, disability, pain, physical activity, exercise capacity, and self-efficacy), and whether fatigue is related to participation in physical education classes, school attendance, and sports frequency. METHODS: The current study used baseline data of 80 patients with JIA (age 8-13) who participated in an intervention aimed at promoting physical activity...

OBJECTIVE: Ictal SPECT is promising for accurate non-invasive localization of the epileptogenic brain tissue in focal epilepsies. However, high quality ictal scans require meticulous attention to the seizure onset. In a relatively large cohort of pediatric patients, this study investigated the impact of the timing of radiotracer injection, MRI findings and seizure characteristics on ictal SPECT localizations, and the relationship between concordance of ictal SPECT, scalp EEG and resected area with seizure freedom following epilepsy surgery...

There are certain disorders of hyper- and hypopigmentation that are more common in children with skin of color. It is imperative that practitioners can swiftly and accurately diagnose these pigmentary disorders to prevent delay in treatment and enhance quality of life. Pigmentary disorders have many etiologies and may present similarly in the clinical setting; however treatment varies widely and is dependent on accurate diagnosis. Herein, we present a review of common disorders of hyperpigmentation and hypopigmentation in children with skin of color...

PURPOSE: Medulloblastoma is the most prevalent childhood brain cancer. Children with medulloblastoma typically receive a combination of surgery, radiation, and chemotherapy. The survival rate is high but survivors often have sequelae from radiotherapy of the entire developing brain and spinal cord. Ongoing genetic studies have suggested that decreasing the dose of radiation might be possible among children with favorable molecular variants; however, this may result in an increased disease recurrence...

OBJECTIVES: Long bone fractures (LBFs) are among the most frequent traumatic injuries seen in emergency departments. Reduction and immobilization is the most common form of treatment for displaced fractures. Point-of-care ultrasound (PoCUS) is a promising technique for diagnosing LBFs and assessing the success of reduction attempts. This article offers a comprehensive review of the use of PoCUS for the diagnosis and reduction of LBFs. Data source MEDLINE and EMBASE databases were searched through July 19, 2015...

BACKGROUND: No consensus exists on management of children with a negative trauma CT following blunt abdominal trauma (BAT). Asymptomatic children are frequently "admitted for observation" following negative CT owing to concerns about missing an intraabdominal injury (IAI) without evidence for this practice. We aimed to investigate the feasibility of discharge following a negative CT scan in children sustaining blunt abdominal trauma. METHODS: Retrospective audit at a UK paediatric major trauma center and review of the literature...

BACKGROUND: To investigate the association between weight status and health-related quality of life (HRQOL) among pupils in Guangzhou, China. METHODS: The study comprised 5781 children aged 8-12 years from 29 schools. Height and weight were objectively measured using standardized methods, and BMI z-score derived using the age and sex specific WHO reference 2007 for 5-19 years. Weight status was classified as underweight (<-2SD), healthy weight (between -2SD and 1SD), overweight/obesity (>1SD)...

Atopic dermatitis (AD) is a common, chronic skin inflammatory disorder characterized by perivascular infiltration of immunoglobulin E (IgE), T lymphocytes, and mast cells. The key factors responsible for the pathophysiology of this disease are immunological disorders and defects in epidermal barrier properties. Pruritus, intense itching, psychological stress, deprived physical and mental performance, and sleep disturbance are the hallmark features of this dermatological disorder. Preventive interventions such as educational programs, avoidance of allergens, and exclusive care toward the skin could play a partial role in suppressing the symptoms...

BACKGROUND: Patient satisfaction has a positive impact on clinical care. Different strategies, such as questionnaires, have been developed to evaluate and improve patient satisfaction. A validated pediatric anesthesia questionnaire previously showed the importance of perioperative care built upon a relationship of trust between healthcare providers and children and parents, and a comfortable environment in which satisfactory answers are provided. However, the questionnaire was validated in English, and no research exists on the use of satisfaction questionnaires in Spanish...

BACKGROUND: The World Health Organization (WHO) early warning indicators (EWIs) of HIV drug resistance (HIVDR) assess factors at individual ART sites that are known to create situations favourable to the emergence of HIVDR. METHODS: In 2014, the Namibia HIV care and treatment program abstracted the following adult and pediatric EWIs from all public ART sites (50 main sites and 143 outreach sites): On-time pill pick-up, Retention in care, Pharmacy stock-outs, Dispensing practices, and Viral load suppression...

BACKGROUND: Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality indicators for transition in SCD and to determine the optimal timing between the final pediatric visit and the first adult provider visit. PROCEDURE: We conducted a modified Delphi survey to reach a consensus on which quality indicators are most important for a successful transition...

INTRODUCTION: The hospital water supply is a reservoir of a variety of potentially pathogenic microorganisms that can particularly affect children and immunocompromised patients. Potentially pathogenic Microsporidium spp. have been identified in water. Microsporidiosis is an emerging parasitic and opportunistic infection in immunocompromised patients. OBJECTIVE AND METHOD: to describe an outbreak of nosocomial diarrhea due to Microsporidium, species Encephalitozoon intestinalis...